Limited cutaneous systemic sclerosis

SIGNS / SYMPTOMS

Nonsystemic type of scleroderma; does not involve the fingers. Absence of Raynaud phenomenon, GERD, and dilated nailbed capillaries.

SIGNS / SYMPTOMS

Condition may be more common in men. Woody induration of extremity or trunk. Peau d'orange (skin pitting like an orange peel) present. Raynaud phenomenon is unusual and there are no dilated nailbed capillaries. The limb may become worse after exercise. [Figure caption and citation for the preceding image starts]: Eosinophilic fasciitis with peau d'orangeFrom the personal collection of Professor J. Pope; used with permission [Citation ends].

SIGNS / SYMPTOMS

There is a history of at least one magnetic resonance image with gadolinium enhancement. Cobblestone, nodular, indurated plaques of the lower extremities, trunk, feet, and hands are present. Raynaud phenomenon and dilated nailbed capillaries are absent.

SIGNS / SYMPTOMS

Diabetes mellitus or a monoclonal gammopathy may coexist. Usually affects only the skin. Skin is indurated, doughy, and involves the neck, back, and face. Raynaud phenomenon, dilated capillaries, and internal organ involvement is absent.

SIGNS / SYMPTOMS

Associated with paraproteinemias.

Affects the hands, face, and extensor surfaces. Raynaud phenomenon and dilated capillaries at the nailbeds are absent. Skin dermis can break into solid papules. Due to the deposition in the skin there can be reduced range of motion of the involved areas.

SIGNS / SYMPTOMS

Associated with diabetes mellitus. Raynaud phenomenon, nailbed dilated capillaries, and skin tightness proximal to the fingers are absent. Typically both hands are affected. Limited joint mobility due to thickening of the skin and contracture of the fingers, which cannot fully extend or flatten. Rarely, larger joints are affected (generally in more advanced, longstanding diabetes mellitus).

SIGNS / SYMPTOMS

Generalized disorder that can affect any system. Most commonly affects women during their reproductive years. Symptoms and signs may accumulate over time. Raynaud phenomenon is invariably bilateral and occurs in as many as 50% of patients at disease onset, although often predating other features of SLE.

SLE has other features such as photosensitivity, malar rash, and oral ulcers.

SIGNS / SYMPTOMS

An idiopathic inflammatory myopathy characterized by the presence of hallmark cutaneous lesions (e.g., heliotrope rash, Gottron papules). Skin lesions are frequently the initial presenting complaint, with muscle involvement developing later. Some patients have cutaneous involvement alone. Raynaud phenomenon may be present.

SIGNS / SYMPTOMS

Other connective tissue diseases (e.g., mixed connective tissue disease) may demonstrate sclerodactyly. Usually involves only the distal half of the fingers.